Familial mediterranean fever medical therapy: Difference between revisions
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==Medical Therapy== | ==Medical Therapy== | ||
*Pharmacologic medical | *Pharmacologic medical therapy of choice for familial Mediterranean fever is colchicine. | ||
*** | *In case of colchicine resistance, other options include: | ||
** | **NSAIDs | ||
* | **IL-1 blockade agents | ||
'''Colchicine''' | |||
** | *'''Adult''' | ||
** | **1.2-1.8 mg PO daily | ||
***** | **Higher initial doses (up to 2 mg per day) may be indicated in those with: | ||
***Preexisting complications (eg, renal amyloidosis) | |||
***High frequency of attacks | |||
***Longer duration of each attack | |||
***Joint involvement | |||
'''Contraindications''' | |||
'''Specific instructions''' | |||
*'''Pediatric''' | |||
**Children younger than 5 years of age, =< 0.5 mg per day (maximum, 0.6 mg per day) | |||
**Children aged 5 to 10 years of age, 0.5 to 1 mg per day (maximum, 1.2 mg per day) | |||
**Children older than 10 years of age, 1 to 1.5 mg per day (maximum, 1.8 mg per day) | |||
==IL-1-blockade== | ==IL-1-blockade== | ||
A [[systematic review]] of [[Interleukin_1|IL-1]] blockade with [[anakinra]], [[canakinumab]], and [[rilonacept]] found only one randomized controlled trial.<ref name="pmid27110096">{{cite journal| author=van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S| title=Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. | journal=Biologics | year= 2016 | volume= 10 | issue= | pages= 75-80 | pmid=27110096 | doi=10.2147/BTT.S102954 | pmc=4831592 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27110096 }} </ref> | A [[systematic review]] of [[Interleukin_1|IL-1]] blockade with [[anakinra]], [[canakinumab]], and [[rilonacept]] found only one randomized controlled trial.<ref name="pmid27110096">{{cite journal| author=van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S| title=Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. | journal=Biologics | year= 2016 | volume= 10 | issue= | pages= 75-80 | pmid=27110096 | doi=10.2147/BTT.S102954 | pmc=4831592 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27110096 }} </ref> | ||
Revision as of 14:56, 28 May 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Clinical practice guidelines direct treatment[1]: The mainstay of treatment for familial mediterranean fever is medical therapy. 03. "Treatment with colchicine should start as soon as a clinical diagnosis is made"
17. "In protracted febrile myalgia, glucocorticoids lead to the resolution of symptoms; NSAID and IL-1-blockade might also be a treatment option; NSAIDs are suggested for the treatment of exertional leg pain"
Medical Therapy
- Pharmacologic medical therapy of choice for familial Mediterranean fever is colchicine.
- In case of colchicine resistance, other options include:
- NSAIDs
- IL-1 blockade agents
Colchicine
- Adult
- 1.2-1.8 mg PO daily
- Higher initial doses (up to 2 mg per day) may be indicated in those with:
- Preexisting complications (eg, renal amyloidosis)
- High frequency of attacks
- Longer duration of each attack
- Joint involvement
Contraindications Specific instructions
- Pediatric
- Children younger than 5 years of age, =< 0.5 mg per day (maximum, 0.6 mg per day)
- Children aged 5 to 10 years of age, 0.5 to 1 mg per day (maximum, 1.2 mg per day)
- Children older than 10 years of age, 1 to 1.5 mg per day (maximum, 1.8 mg per day)
IL-1-blockade
A systematic review of IL-1 blockade with anakinra, canakinumab, and rilonacept found only one randomized controlled trial.[2]
- anakinra: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 3.5 with placebo to 1.7 with anakinra.[3] Average age was 37 years and 83% of subjects were homozygous for M694V. This trial was published and registered (NCT01705756).
- canakinumab: no randomized controlled trials.
- rilonacept: benefit in one small, randomized controlled trial in which the median number of attacks per month dropped from 2 with placebo to 0.77 with rilonacept.[4] Average age was and 24 years and 7% of subjects were homozygous for M694V This trial was published and registered (NCT00582907).
References
- ↑ Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G; et al. (2016). "EULAR recommendations for the management of familial Mediterranean fever". Ann Rheum Dis. 75 (4): 644–51. doi:10.1136/annrheumdis-2015-208690. PMID 26802180.
- ↑ van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S (2016). "Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature". Biologics. 10: 75–80. doi:10.2147/BTT.S102954. PMC 4831592. PMID 27110096.
- ↑ Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity S; et al. (2017). "Anakinra for Colchicine-Resistant Familial Mediterranean Fever: A Randomized, Double-Blind, Placebo-Controlled Trial". Arthritis Rheumatol. 69 (4): 854–862. doi:10.1002/art.39995. PMID 27860460.
- ↑ Hashkes PJ, Spalding SJ, Giannini EH, Huang B, Johnson A, Park G; et al. (2012). "Rilonacept for colchicine-resistant or -intolerant familial Mediterranean fever: a randomized trial". Ann Intern Med. 157 (8): 533–41. doi:10.7326/0003-4819-157-8-201210160-00003. PMID 23070486.